Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 15  |  Issue : 1  |  Page : 27-33

Management of lower extremity soft-tissue sarcoma in a sub-Saharan African teaching hospital: Case reports


1 Department of Surgery, College of Medicine, University of Ibadan; Department of Surgery, University College Hospital, Ibadan, Nigeria
2 Department of Surgery, College of Medicine, University of Ibadan; Department of Plastic, Reconstructive & Aesthetic Surgery, University College Hospital, Ibadan, Nigeria
3 Department of Surgery, University College Hospital, Ibadan, Nigeria

Date of Web Publication26-Aug-2019

Correspondence Address:
Dr. Samuel A Ademola
Department of Surgery, College of Medicine, University of Ibadan, University College Hospital, PMB 5116, Ibadan
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njps.njps_14_18

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  Abstract 


Background: Soft-tissue sarcomas are relatively rare tumors and can occur in many parts of the body. When they affect the body extremities, their management can be challenging, often leading to limb amputation. Recent advances in surgery, adjuvant therapy, and better collaboration among different surgical specialists, medical and radiation oncologists, coupled with management in specialized centers have led to an upsurge in limb preservation. However, this is not obtainable in many centers in the developing countries. We report cases of soft-tissue sarcoma of the lower limb in two patients managed in a tertiary center in sub-Saharan Africa and the challenges encountered in their management. Case Reports: Two patients presented to our hospital with progressive painless masses on the lower third of their legs. Evaluation suggested that the masses were malignant. They both had wide local excision. The first patient had reconstruction with island sural artery fasciocutaneous flap, whereas the second had reconstruction with freestyle propeller flap. The postoperative periods were uneventful, and timely adjuvant therapy was commenced. Limb function was preserved in the two patients. Conclusion: Treatment of soft-tissue sarcomas of the limbs could be tasking, but multispecialty surgical intervention and adequate adjuvant therapy could give favorable result and a functional limb postoperatively.

Keywords: Extremity, flaps, soft-tissue sarcoma, sub-Saharan Africa


How to cite this article:
Ayandipo OO, Ademola SA, Afuwape OO, Michael AI, Elemile PO, Udonsak NS. Management of lower extremity soft-tissue sarcoma in a sub-Saharan African teaching hospital: Case reports. Nigerian J Plast Surg 2019;15:27-33

How to cite this URL:
Ayandipo OO, Ademola SA, Afuwape OO, Michael AI, Elemile PO, Udonsak NS. Management of lower extremity soft-tissue sarcoma in a sub-Saharan African teaching hospital: Case reports. Nigerian J Plast Surg [serial online] 2019 [cited 2019 Sep 16];15:27-33. Available from: http://www.njps.org/text.asp?2019/15/1/27/265405




  Introduction Top


Soft-tissue sarcomas are rare and heterogeneous group of tumors, representing less than 1% of all adult malignancies.[1] Sixty percent of soft-tissue sarcomas occur in the extremities, making this the most common site.[1] Other locations are the trunk (19%), the retroperitoneum (15%), and the head and neck (9%).[1] When feasible, function-sparing surgical excision with wide margins is the cornerstone of effective treatment, the goal being the preservation of a functional extremity, as there is no significant difference in disease-free survival (DFS) and overall survival (OS) when comparing limb-sparing surgery with amputation.[2],[3]

Although limb amputation may be unavoidable in some circumstances, to obtain local control and offer the best chance of cure, the combination of limb-sparing technique and reconstructive surgery can optimize function of the affected limb and avoid the significant psychological impact associated with amputation.[4]

Currently, 90% to 95% of patients with limb sarcomas may undergo successful limb-sparing procedures with soft-tissue coverage when treated at major centers specialized in musculoskeletal oncology.[5] Such centers, often located in the developed parts of the world, offer care overseen by a team of specialists, which include surgical oncologists, plastic surgeons, medical and radiation oncologists, pathologists, radiologists, and ancillary staff. They are also not constrained by a lack of resources for adequate patient care and so are able to offer better adjuvant treatment options and specialized reconstructions. Conversely, resources and expertise are lacking in many centers in developing countries and surgeons are sometimes forced to resort to amputations due to infrastructural hurdles.[6],[7]

Hence, we report successful management of two cases of soft-tissue sarcoma of the lower limbs.


  CASE 1 Top


A 32-year-old man presented to the surgery outpatient clinic with a rapidly growing, painless left leg swelling of 6 months.

On examination, he had a 10 cm × 6 cm × 4 cm swelling on the anterolateral aspect of the left leg arising 6 cm above the lateral malleolus. The surface was ulcerated, and the surrounding skin was hyperpigmented [Figure 1]. The mass was nontender and hard with reduced mobility on dorsiflexion. There was no peripheral lymphadenopathy. He was presented at the oncoplastic multidisciplinary team (MDT) meeting and scheduled for a wide local excision and immediate soft-tissue reconstruction, and planned for adjuvant external beam radiotherapy thereafter.
Figure 1 Ulcerated mass on lateral aspect of the leg

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Fine-needle aspirate cytology was equivocal. It has been our practice to do this in our patients instead of core biopsy because of its ease and high correlation in patients with soft-tissue sarcoma.

Abdominal ultrasonography and chest and limb radiographs were done because the patient could not afford computerized tomography, and these did not reveal evidence of metastasis or bony involvement.

Intraoperative findings were of a tumor arising from the belly of the tibialis anterior muscle. This was resected with a margin of normal tissue leaving all flexor tendons intact [Figure 2]. Immediate reconstruction was performed using a reversed sural artery flap and split thickness skin grafting of the secondary defect [Figure 3].
Figure 2 Primary defect showing flexor tendons intact

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Figure 3 Defect reconstructed with reversed sural artery flap

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Histology showed an intermediate-grade fibrosarcoma with negative resection margins.


  CASE 2 Top


A 26-year-old male presented to the surgery outpatient clinic with a painless, progressively increasing right lower leg mass of 5 months.

On examination, he had a 10 cm × 10 cm × 4 cm hemispherical mass on the anterolateral aspect of the right leg, 3 cm above the lateral malleolus [Figure 4]. The mass was nontender, hard, and slightly mobile irrespective of joint position.
Figure 4 Mass located 3 cm above the lateral malleolus

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Fine-needle aspirate cytology was suggestive of a malignant lesion.

Abdominal ultrasonography and chest and limb radiographs did not reveal any bony involvement or metastasis. He was presented at the oncoplastic MDT meeting and scheduled for a wide local excision with soft-tissue cover and adjuvant external beam radiotherapy.

Intraoperative findings were of a soft-tissue tumor attached to the skin, which was resected down to the periosteum of the fibula [Figure 5] and [Figure 6]. Immediate reconstruction was performed using a peroneal artery perforator-based propeller flap and split thickness skin grafting of the secondary defect [Figure 7].
Figure 5 Mass after wide local resection

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Figure 6 Primary defect showing depth of resection down to periosteum of the fibula

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Figure 7 Defect after reconstruction with propeller flap

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Histology showed an intermediate-grade dermatofibrosarcoma protuberans with involvement of the deep margins of resection.


  Discussion Top


Soft-tissue sarcomas commonly present as a painless, slow-growing mass. Although sarcomas in the extremities may present earlier, diagnosis of sarcomas involving the pelvic cavity may be delayed as their location deep within the body precludes palpation of the tumor mass early during the disease. Consequently, these tumors often attain enormous sizes prior to diagnosis without causing overt symptoms. Any soft-tissue lump exhibiting any of the following four clinical features should be malignant until proved otherwise: (i) increasing in size, (ii) size >5 cm, (iii) location deep to the deep fascia, or (iv) pain. The more the number of these clinical features present, the greater the risk of malignancy, with increasing size being the best individual indicator.[4] Tumors in the distal extremities are often smaller when discovered, giving the opportunity for earlier and potentially curative therapy.[8] It is also easier to administer adjuvant radiation therapy and to follow-up for local recurrence.

When feasible, function-sparing surgical excision with wide margins is the cornerstone of effective treatment, the goal being the preservation of a functional extremity, while limiting the chances of local recurrence by excising as wide a margin as concomitant neurovascular structures will permit.[9] This may be facilitated by soft-tissue reconstructive surgery, including the use of local or free flaps and, occasionally, vascular and nerve resection with suitable graft reconstruction. Surgery for these lesions is best performed by specialist surgeons who practice in high-volume centers.[10] Patients should have their care managed by a formally constituted sarcoma MDT. It is this team that makes decisions about surgery, chemo-radiation, and the timing of all these modalities.[4] However, few centers in West Africa have these specialist teams and the required resources for these surgical procedures. The availability of such specialists in our center made it possible to manage these cases.

The standard surgical procedure is a wide excision. This implies removal of the tumor along with a rim of surrounding normal tissue in all cases. On occasion, anatomical constraints in the extremities mean that a true wide resection is not possible without the sacrifice of critical anatomical structures (such as major nerves, blood vessels, or bone), and in this situation, a marginal excision may be acceptable as an individualized option in highly selected cases, after having considered the risks of recurrence and the morbidity of more radical surgery and after full discussion of these factors with the patient.[2] Limb-sparing surgery is successful when the limb left behind is useful to the patient. Both the reported cases had a wide local excision with one of the patients having negative microscopic margins and the other having positive microscopic margins but with preserved limb function. This is in line with the practice recommendations that to preserve functionality, surgery may result in a close or even a microscopically positive margin and postoperative radiation should be considered.[11],[12]

As tumor excision often leads to potentially large tissue defects, including bone, joint, and tendon exposure, reconstructive surgery is an important and critical element of management.[7] Local anatomy at the tumor site also has significant influence on the ease of surgical reconstruction. The two patients presented had tumors located in the distal third of the leg. This part of the body presents a lot of challenges for reconstruction after wide excision for many reasons. The blood supply of the distal part of the leg is relatively poor due to unreliable subdermal plexus leading to poor healing when cutaneous flaps are raised, and muscles become tendinous, therefore cannot provide the bulk required for coverage of defects.[13],[14] In addition, both the tibia and fibula are subcutaneous as they become the medial and lateral malleolus, and there is limited laxity of the skin of this region.[15],[16] All these limit available options of reconstruction and may influence the adequacy of excision margins. Inadequate surgical excision could be prevented by undertaking the surgery with a reconstructive surgeon who has a vast experience in reconstructions of the lower limbs and by the use of frozen section biopsies during surgery. Frozen section assists the surgeon in determining the adequacy of oncologic resection margins.[17] This technology is a challenge in a developing country such as ours. Neither of our patients had frozen section, which could have been helpful in the second patient who still had positive margins.

Free-tissue transfer has been the main method of reconstruction after surgical resection of soft-tissue sarcomas in the distal part of the leg and foot due to the reasons adduced earlier. As a result of this, many patients in the practice setting of this report undergo limb amputation because of the dearth of expertise and equipment required for this method. Free-tissue transfer requires specialized training, entails a steep learning curve, sophisticated equipment, and prolonged operative time with attendant anesthetic challenges.[13],[14] These requirements are often not commonly available in our practice setting and make this option of management out of reach of patients. More recently, better understanding of lower limb anatomy, advances in wound care, and better orthopedic fixation methods have allowed for use of other reconstructive methods rather than free flaps, therefore leading to avoidance of the difficulties associated with free-tissue transfers.[18]

Limb salvage was possible in these two patients because of the availability of local flaps, and this precluded the mandatory use of free flaps without which an amputation would have been necessary. Reversed sural artery flap reconstruction used in reconstruction of the first patient’s wound is a method that can be employed to overcome the challenges posed by anatomical and surgical difficulties in the patients and infrastructural deficiencies that attends their management. It also allows for immediate reconstruction after surgical excision. In the second patient, the vascular pedicle of the sural artery flap was involved in the wide excision; therefore, it was not available for reconstruction. A propeller flap has been proposed for such demanding situations when the vascular basis of sural artery flap is involved in tumor excision or trauma,[18] and this knowledge was utilized for postexcision reconstruction in the patient. There are several advantages for immediate reconstruction to be carried out at the time of tumor resection. One is that the anatomical perspective of the oncological defect can be assessed prior to scar formation. This will minimize surgical dissection to expose vessels for microvascular repair that would be necessitated if there was a delay with scar formation. Another advantage is the psychological benefit to the patient.[11] The two cases we are reporting had large tissue defects and had immediate flap reconstruction.

The presence of a soft-tissue sarcoma in the extremity is no longer an indication for amputation.[11] The goals of sarcoma management include both a cure and functional preservation of involved tissues and adjacent critical structures. This line of management has incidentally coincided with improved OS rates of soft-tissue sarcoma patients with no significant difference in DFS.[2],[3],[19] Microscopically, positive margins of resection are not necessarily associated with local recurrence or poor prognosis, especially when adjuvant radiotherapy is utilized.[3],[10] Radiotherapy can be administered both intraoperatively as external beam radiotherapy or brachytherapy and postoperatively. Advantages of intraoperative radiotherapy include reduced dose and volumes of radiation with better tolerance of normal tissue. Postoperatively, radiation therapy should be administered with the best technique available at a dose of 50 to 60 Gy,[20] and this modality is what is available in our setting.

Data suggest that adjuvant chemotherapy may delay distal or local recurrence in high-risk patients. However, studies have been conflicting, and adjuvant chemotherapy is not standard in adult-type soft-tissue sarcomas.[20] The patients in our report did not receive chemotherapy.Limb loss from congenital deficiency and surgical or traumatic severance of extremity is a devastating condition.[21] Major lower limb amputation results in significant global morbidity and mortality.[22] Apart from physical disability, loss of a limb of any individual causes profound economic, social, and psychological effect on the patients and their families. This effect is more pronounced in developing countries where prosthetic services are not readily available.[22],[23],[24] Indeed, many patients in our environment would default treatment once the option of amputation is offered. In the cases reported, our patients were satisfied with the functional outcome and adjuvant radiotherapy. This was made possible because of the availability of a specialist MDT in our center and access of these two patients to postoperative radiotherapy.


  Conclusion Top


The treatment of large soft-tissue sarcomas of the extremity should include a combination of surgical intervention and external beam radiotherapy. Function-sparing surgical excision with wide margins followed by reconstruction can give favorable outcomes.

This is possible in developing countries, especially in the presence of a specialist MDT and with availability of postoperative radiation therapy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Olawoye OA, Ademola SA, Iyun AO, Michael A, Oluwatosin OM. The reverse sural artery flap for the reconstruction of distal third of the leg and foot. Int Wound J 2014;11:210-4.  Back to cited text no. 14
    
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de Blacam C, Colakoglu S, Ogunleye AA, Nguyen JT, Ibrahim AMS, Lin SJ et al. Risk factors associated with complications in lower-extremity reconstruction with the distally based sural flap: A systematic review and pooled analysis. J Plast Reconstr Aesthet Surg 2014;67:607-16.  Back to cited text no. 15
    
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Reece GP, Schusterman MA, Pollock RE, Kroll SS, Miller MJ, Baldwin BJ et al. Immediate versus delayed free-tissue transfer salvage of the lower extremity in soft tissue sarcoma patients. Ann Surg Oncol 1994;1:11-7.  Back to cited text no. 24
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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